Clinical features and prognosis of autoimmune pancreatitis alone or with IgG4-related sclerosing cholangitis / 临床肝胆病杂志

ABSTRACT

ObjectiveTo investigate the clinical features, diagnosis, treatment, and prognosis of autoimmune pancreatitis (AIP) alone versus AIP with IgG4 sclerosing cholangitis (IgG4-SC). MethodsA retrospective analysis was performed for the clinical data of 40 patients with type 1 AIP who were admitted to The First Affiliated Hospital of Zhengzhou University from June 2015 to January 2020, among whom 29 patients had AIP alone and 11 had AIP with IgG4-SC. The two groups were compared in terms of clinical manifestations, laboratory examination, imaging findings, treatment, and prognosis. The t-test was used for comparison of normally distributed continuous data between two groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups; the Fisher’s exact test was used for comparison of categorical data between two groups. The Kaplan-Meier method was used to calculate recurrence rate and plot recurrence curve, and the log-rank test was used for univariate analysis. ResultsCompared with the AIP group, the AIP+IgG4-SC group had significantly higher number of affected organs ［3.0(3.0-4.0) vs 3.0(1.5-3.5), Z=-2.172, P=0035］ and response index before treatment ［12.0(12.0-15.0) vs 12.0(9.0-13.5), Z=-2.157, P=0.032］. The AIP+IgG4-SC group had a significantly higher median serum IgG level than the AIP group ［21.0(15.8-23.7) g/L vs 14.8(13.3-15.7) g/L, Z=-2.711, P=0.004］. During the median follow-up time of 15.8 (6.5-31.3) months, the AIP+IgG4-SC group had a significantly higher recurrence rate than the AIP group (χ2=8.155, P=0.004). ConclusionPatients with AIP and IgG4-SC tend to have higher serum IgG4 level, number of affected organs, and recurrence rate than those with AIP alone. Early identification, diagnosis, and treatment can reduce the recurrence rate of AIP.